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Nutrition Management of Glycogen Storage Disease Type 1

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Book cover Nutrition Management of Inherited Metabolic Diseases

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Abstract

  • Glycogen storage disease types 1a and 1b (GSD-1) are characterized by fasting hypoglycemia and elevated lactic acid, uric acid, cholesterol, and triglycerides. Patients with GSD type 1b are also at risk of neutropenia and inflammatory bowel disease.

  • Nutrition management of GSD-1 includes providing supplemental uncooked cornstarch as a source of glucose, avoidance of dietary galactose and fructose, and a moderate restriction of fat.

  • Depending on age, overnight continuous feeding or uncooked cornstarch feedings every 4–6 h are necessary to prevent hypoglycemia during the night.

  • Frequent home monitoring of blood glucose and adjustments in the diet are needed to prevent hypoglycemia and maintain blood glucose >70 mg/dL or 4 mmol/L.

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Authors and Affiliations

  1. Department of Molecular and Medical Genetics and Graduate Programs in Human Nutrition, School of Medicine, Oregon Health and Science University, 3181 S.W. Sam Jackson Park Rd., Portland, OR, 97239-3098, USA

    Sandy van Calcar PhD, RDN, LDN

Authors
  1. Sandy van Calcar PhD, RDN, LDN
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Correspondence to Sandy van Calcar PhD, RDN, LDN .

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Editors and Affiliations

  1. Clinical Genetics and Metabolism, Children’s Hospital Colorado, University of Colorado Denver – Anschutz Medical Campus, Aurora, Colorado, USA

    Laurie E. Bernstein

  2. Division of Genetics and Genomics, Boston Children’s Hospital, Boston, Massachusetts, USA

    Fran Rohr

  3. Clinical Genetics and Metabolism, University of Colorado Denver, Children’s Hospital Colorado, Aurora, Colorado, USA

    Joanna R. Helm

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© 2015 Springer International Publishing Switzerland

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van Calcar, S. (2015). Nutrition Management of Glycogen Storage Disease Type 1. In: Bernstein, L., Rohr, F., Helm, J. (eds) Nutrition Management of Inherited Metabolic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-14621-8_27

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  • DOI: https://doi.org/10.1007/978-3-319-14621-8_27

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-14620-1

  • Online ISBN: 978-3-319-14621-8

  • eBook Packages: MedicineMedicine (R0)

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